["Benign" monoclonal gammopathy and chronic lymphatic leukemia in a patient with Noonan syndrome]
- PMID: 9711057
- DOI: 10.1007/BF03042640
["Benign" monoclonal gammopathy and chronic lymphatic leukemia in a patient with Noonan syndrome]
Abstract
History: At the age of 32 a "benign" monoclonal gammopathy of lightchain kappa with Bence Jones protein is diagnosed in a man born 1934. In addition a Noonan-syndrome is found.
Course: Twenty-four years later he gradually develops a chronic lymphatic leukaemia (B-CLL) which up to now does not need treatment (October 1996). The neoplastic B-cells exprime monoclonal lightchain lambda on the cellmembrane and in the cytoplasma undetectable by immunefixation in the serum. Irrespective of that the known monoclonal gammopathy exprimes IgG-kappa without an increase in the number of plasmacells in the bonemarrow.
Conclusion: There are hints that the congenital Noonan-syndrome can be associated with B-cell disorders.
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